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J Thorac Cardiovasc Surg 2003;125:733-735
© 2003 The American Association for Thoracic Surgery
Brief Communications |
From the Section of Cardiology, Department of Medicine,a the Section of Cardiac Surgery, Department of Surgery,b the Division of Anatomical Pathology, Department of Pathology and Laboratory Medicine,c and the Department of Anatomy and Neurobiology,d Dalhousie University, Halifax, Nova Scotia, Canada.
Received for publication July 12, 1001. Accepted for publication July 22, 2002. Address for reprints: Davinder S. Jassal, MD, FRCPC, Department of Cardiology, Queen Elizabeth II Health Sciences Centre, Room 2134-1796 Summer St, Halifax, Nova Scotia, Canada B3K 6A3 (E-mail: umjassal{at}hotmail.com).
A 49-year-old woman with a history of mitral valve prolapse presented with pleuritic chest discomfort of 2 weeks' duration. During the follow-up period, echocardiography and magnetic resonance imaging (MRI) confirmed a right atrial mass measuring 5 x 3 cm. After cardiopulmonary bypass, the histologic and immunohistochemical features were consistent with an ancient cardiac schwannoma. To the best of our knowledge, there have been only 5 previous cases of primary cardiac schwannomas described in the literature, all presenting with symptomatic pericardial effusions; our case was notable for its rather benign presentation.
Clinical summary
A 49-year-old woman with a history of mitral valve prolapse presented with a 2-week history of episodic pleuritic chest discomfort. She denied symptoms of exertional dyspnea, orthopnea, palpitations, syncope, or infection or any risk factors for thromboembolism.
The patient was afebrile and normotensive and had no signs of systemic infection. The cardiorespiratory examination was remarkable only for signs of mitral valve prolapse with mild mitral regurgitation. There was no calf or thigh swelling or tenderness.
The complete blood count, electrolyte level, liver function test results, cardiac enzyme level, coagulation parameters, and urinalysis results were within normal limits. Electrocardiography revealed sinus bradycardia with a normal chest radiograph. An echocardiographic examination revealed mild prolapse of the anterior mitral leaflet, mild mitral regurgitation, and a mass measuring 5 x 3 cm in size occupying the entire right atrium. Additionally, MRI confirmed a right atrial mass of 5 x 3 cm (Figure 1). These diagnostic tests were unable to discern whether the mass was intracavitary or extrinsic in nature. Mammography, bone scan, and infused computed tomography of the abdomen and pelvis were also performed to rule out metastatic malignant disease; results of these studies were normal. A coronary catheterization revealed normal coronary vasculature.
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, B). On microscopy, the tumor consisted of spindled tumor cells with an eosinophilic cytoplasm and elongated nuclei, some demonstrating a wavy appearance (Figure 3, A). Histologic and immunohistochemical features were consistent with an ancient schwannoma (Figure 3, B).
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Primary cardiac tumors are an extremely rare diagnostic entity. 1 The clinical outcome depends not only on the histology of the tumor but also on its location and rate of growth in addition to how promptly it is detected and appropriately treated. Although the first symptoms are often constitutional, cardiovascular symptoms, including dyspnea, orthopnea, dependent edema, and thromboembolism, are often the presenting manifestations. 1,2
Although left atrial myxomas are by far the most common primary cardiac tumors diagnosed, our patient presented with a rather unique ancient cardiac schwannoma. The nervous system of the heart resides in the epicardial fat associated with the atria and ventricles and contains both myelinated and unmyelinated axons. 3 Associated neurons are thus capable of undergoing pathologic and functional changes in cardiac disease, as in our patient. A MEDLINE review of the literature published between 1966 and 2002 revealed only 5 previously described cases of primary cardiac schwannomas, all of which presented with symptomatic pericardial effusions; our case was notable for its rather benign presentation. 2-5
The diagnosis of a primary cardiac neoplasm is best screened by means of transthoracic echocardiography. Whereas the identification of endocardial-based lesions in the atria is best delineated with transesophageal echocardiography, the assessment of pericardiac masses remains the strength of MRI. 1 Once a diagnosis is made, the patient should have prompt removal of the cardiac neoplasm after an appropriate work-up to rule out metastatic disease of the heart and pericardium. The first step toward the appropriate diagnosis of a cardiac neoplasm remains when the clinician considers it in the differential diagnosis.
References
This article has been cited by other articles:
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  S. La Francesca, I. D. Gregoric, W. E. Cohn, and O.H. Frazier Successful Resection of a Primary Left Ventricular Schwannoma Ann. Thorac. Surg., May 1, 2007; 83(5): 1881 - 1882. [Abstract] [Full Text] [PDF]
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